Mixed neuroendocrine–nonneuroendocrine neoplasms of the gastrointestinal system:An update

Mixed neuroendocrine-nonneuroendocrine neoplasms(Mi NENs)of the digestive tract are a rare heterogeneous group of tumors that present many challenges in terms of diagnosis and treatment.Over the years,the diagnostic criteria,classification,and clinical behavior of these tumors have been the subjects of ongoing debate,and the various changes in their nomenclature have strengthened the challenges associated with Mi NENs.This review is performed to provide an understanding of the key factors involved in the evolution of the designation of these tumors as Mi NEN,highlight the current diagnostic criteria,summarize the latest data on pathogenesis and provide information on available treatments.Moreover,this work seeks to increase the awareness about these rare neoplasms by presenting the clinicopathological features and prognostic factors that play important roles in their behavior and discussing their different regions of origin in the gastrointestinal system(GIS).Currently,the Mi NEN category also includes tumors in the GIS with a nonneuroendocrine component and epithelial tumors other than adenocarcinoma,depending on the organ of origin.Diagnosis is based on the presence of both morphological components in more than 30%of the tumor.However,this value needs to be reconfirmed with further studies and may be a limiting factor in the diagnosis of Mi NEN by biopsy.Furthermore,available clinicopathological data suggest that the inclusion of amphicrine tumors in the definition of Mi NEN is not supportive and warrants further investigation.The diagnosis of these tumors is not solely based on immunohistochemical findings.They are not hybrid tumors and both components can act independently;thus,careful grading of each component separately is required.In addition to parameters such as the metastatic state of the tumor at the time of diagnosis and the feasibility of surgical resection,the aggressive potential of both components has paramount importance in the choice of treatment.Regardless of the organ of origin within the GIS,almost Mi NENs are tumors with poor prognosis and are frequently encountered in the elderly and men.They are most frequently reported in the colorectum,where data from molecular studies indicate a monoclonal origin;however,further studies are required to provide additional support for this origin.

胰腺混合性腺泡-神经内分泌癌的临床病理特征分析

目的探讨胰腺混合性腺泡-神经内分泌癌(MAEC)的临床病理特征。方法分析1例胰腺混合性腺泡-神经内分泌癌的临床表现、病理形态学特征及免疫组化特点,并结合文献进行讨论。结果患者男性,54岁。无明显诱因出现乏力,伴纳差,偶有反酸嗳气、腹胀。CT及MRI检查提示胰尾部、脾门区及脾实质内占位性病变,边界不清。行胰腺体尾部、脾及部分胃切除术。肿瘤组织由腺泡细胞癌及神经内分泌肿瘤两种组织学形态组成,两种成分穿插混合生长。神经内分泌肿瘤CD56、Cg A和Syn(+);腺泡细胞癌亦CD56和Syn(+);但仅散在个别细胞Cg A(+)。腺泡状区域bcl-10和CAM5.2弥漫性强(+),而梁索状区域弱(+)。Ki-67增殖指数均为3%。术后给予化疗,8个月后出现肝转移,目前随访15个月,患者一般状态良好。结论胰腺混合性腺泡-神经内分泌癌非常罕见,易与胰腺腺泡细胞癌、神经内分泌肿瘤、胰腺实性假乳头状肿瘤及胰母细胞瘤等混淆。

胰腺混合性腺泡-神经内分泌癌1例并文献复习

目的探讨胰腺混合性腺泡-神经内分泌癌(MANEC)的诊断和治疗。方法回顾性分析昆明医科大学第二附属医院收治的1例MANEC患者临床资料,结合国内外文献,对患者的发病情况、临床表现、病理、影像学特点、诊断及治疗等进行分析。结果该病多见于中老年男性患者,确诊需病理检查,B超、CT或磁共振成像检查后提示占位性病变考虑胰腺癌,行胰腺切除,胰腺、空肠吻合术。术后病检确诊为MANEC,术后患者治愈出院。结论影像学检查可协助诊断MANEC,病理组织活检是诊断该病的金标准,外科手术是唯一可根治MANEC的治疗方法。

胃肠胰腺癌伴神经内分泌分化和胃肠胰混合性腺-神经内分泌癌的临床病理研究

目的探讨胃肠胰腺癌伴神经内分泌分化（NED）和胃肠胰混合性腺-神经内分泌癌（MANEC）的临床病理特征。方法回顾性分析浙江省中医院29例胃肠胰腺癌伴神经内分泌分化和7例胃肠胰MANEC患者的临床病理资料，对其组织学苏木精-伊红染色（HE）及免疫组织化学染色（IHC）进行观察，比较IHC指标及肿瘤远处转移与临床病理指标间的关系。结果胃肠胰腺癌伴NED和MANEC患者各临床病理指标和IHC表达率之间差异无统计学意义；神经内分泌指标表达阳性率与临床病理因素之间差异无统计学意义；远处转移与临床病理因素之间差异无统计学意义。结论结合形态学HE染色和免疫组化IHC染色胃肠胰腺癌伴NED和MANEC具有很高的异质性,临床分期及分级是主要的预后因素,早期诊断及治疗可改善患者预后。